Chintagumpala M M, Hurwitz R L, Moake J L, Mahoney D H, Steuber C P
Department of Pediatrics (Hematology/Oncology), Baylor College of Medicine, Houston, TX.
J Pediatr. 1992 Jan;120(1):49-53. doi: 10.1016/s0022-3476(05)80596-7.
We studied two children with recurrent schistocytic hemolytic anemia and thrombocytopenia beginning in the neonatal period. One patient had a stroke during one of the episodes of thrombotic thrombocytopenic purpura. The presence of unusually large von Willebrand factor multimers was demonstrated in both children during clinical and hematologic remissions. Treatment with corticosteroids and intravenous injections of immune globulin was unsuccessful in the one patient who received it. Immediate improvement occurred in both patients after the infusion of fresh-frozen plasma. Symptoms of thrombocytopenia continue to recur at regular intervals in the absence of periodic fresh-frozen plasma infusions. One of these children apparently has chronic relapsing thrombotic thrombocytopenic purpura; the second has a chronic relapsing disorder similar to thrombotic thrombocytopenic purpura.
我们研究了两名自新生儿期起就患有复发性裂体细胞性溶血性贫血和血小板减少症的儿童。其中一名患者在血栓性血小板减少性紫癜发作期间发生了中风。在两名儿童临床和血液学缓解期均证实存在异常大的血管性血友病因子多聚体。接受皮质类固醇和静脉注射免疫球蛋白治疗的那名患者治疗无效。两名患者在输注新鲜冷冻血浆后均立即出现改善。在没有定期输注新鲜冷冻血浆的情况下，血小板减少症症状会定期复发。其中一名儿童显然患有慢性复发性血栓性血小板减少性紫癜；另一名患有与血栓性血小板减少性紫癜相似的慢性复发性疾病。
